Evidence map›Paper›PMID 40971335›Full record

SynthesisNephron2026

Clinical Presentation, Treatment Patterns, Burden of Disease, and the Association of Proteinuria with Clinical Outcomes in C3 Glomerulopathy and Primary Immune Complex Membranoproliferative Glomerulonephritis: A Systematic Review.

Fernando Caravaca-Fontán, Fadi Fakhouri, Matthew C Pickering, Vikte Lionikaite, Alison Baird, Regina Horneff, Luis López-Lázaro, Lucia Quintana-Gallardo, Carly Rich

Abstract readSystematic Review
In one paragraph

Synthesis in Nephron, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Article
  2. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors.

Fernando Caravaca-FontánDepartment of Nephrology, Instituto de Investigación Hospital 12 de Octubre (imas12), Madrid, Spain.
Fadi FakhouriService of Nephrology and Hypertension, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
Matthew C PickeringDepartment of Immunology and Inflammation, Imperial College London, London, UK.
Vikte LionikaiteOxford PharmaGenesis, Oxford, UK.
Alison BairdOxford PharmaGenesis, Oxford, UK.
Regina HorneffSwedish Orphan Biovitrum AB, Stockholm, Sweden.
Luis López-LázaroSwedish Orphan Biovitrum AB, Stockholm, Sweden.
Lucia Quintana-GallardoSwedish Orphan Biovitrum AB, Stockholm, Sweden.
Carly RichSwedish Orphan Biovitrum AB, Stockholm, Sweden, carly.rich@sobi.com.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionKnowledge gaps and controversies remain regarding the natural history and variability of C3 glomerulopathy (C3G) and primary immune complex membranoproliferative glomerulonephritis (IC-MPGN). The objectives were to provide an overview of these diseases for the following outcomes of interest: clinical presentation, treatment patterns, and disease burden, including the association between proteinuria and kidney outcomes.

methodsThis systematic literature review (SLR) included studies of adults and children with C3G or primary IC-MPGN investigating outcomes of interest. Embase and MEDLINE were searched from January 2012 to February 22, 2024, combining terms for C3G or IC-MPGN and outcomes of interest. Supplementary congress searches and reference list checking of relevant articles were conducted. Study details, outcomes of interest, and key findings were extracted, and data were narratively summarized.

resultsIn total, 148 articles were included. No clear trend for differences between C3G and primary IC-MPGN were observed for clinical presentation. Treatments included immunosuppressive therapies and off-label anti-complement agents. Kidney failure occurred in up to 50% and 37% of patients with C3G and primary IC-MPGN, respectively, and kidney transplantation was required in up to 32% and 24% of patients, respectively. Mortality was reported in up to 21% of patients. No clear trend of complete remission across treatments was observed. In longitudinal studies, proteinuria was associated with increasing risk of kidney failure. No articles reported on patient quality of life or caregiver burden. Several articles reported an economic burden according to length of hospital stay. Possible limitations include that terms used for electronic searches limited which articles were identified, many studies were retrospective and small (<10 participants), and risk of bias was not performed.

conclusionsThis SLR provides insights into C3G and primary IC-MPGN, emphasizing the need for new targeted and effective treatments. Proteinuria was identified to be an acceptable marker in assessing the efficacy of treatments on long-term kidney outcomes.

Indexed as

Complement C3Glomerulonephritis, MembranoproliferativeProteinuriaCost of IllnessHumansTreatment OutcomeComplement C3C3 glomerulopathyDisease burdenImmune complex membranoproliferative glomerulonephritisProteinuriaSurrogate marker

Identifiers

PMID40971335
PMCPMC12795539

What OpenQuestion holds

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.